【摘要】 目的 探索先天性束带综合征手部畸形的表现，以丰富对该病手部畸形的认识，指导临床评估和治疗。方法 回顾 2012 年 1 月至 2024 年 1 月，于本中心诊治的束带综合征患儿，记录年龄、性别、手部及手指受累情况和合并畸形，根据临床资料和影像资料评估指骨发育不全、窄缩带和并指的畸形表现及严重程度并据此进行分类。结果 本研究共纳入 53 例，男 23 例 (43.40%)，女 30 例 (56.60%)，年龄 0.92～17 岁，平均 (3.92±4.01) 岁。双手受累 27 例，单纯左手受累 11 例，右手受累 14 例，前臂受累但手部未受累1 例。530 指中，Ⅰ 类指骨发育不全 69 指 (13.02%)，Ⅱ 类 153 指 (28.87%)，Ⅲ 类 52 指 (9.81%)。530 指中，142 指 (21.87%) 存在窄缩带畸形，Ⅰ 类占 34 指 (6.42%)，Ⅱ 类 41 指 (7.74%)，Ⅲ 类 24 指 (4.53%)，Ⅳ 类 28 指 (5.28%)，多发束带 17 指 (3.21%)。212 个指蹼中，Ⅰ 类并指 10 指蹼 (1.89%)，Ⅱ 类 46 指蹼(8.68%)，Ⅲ 类 12 指蹼 (2.26%)，Ⅳ 类 16 指蹼 (3.02%)，Ⅴ 类 9 指蹼 (1.70%)，Ⅵ 类 4 指蹼 (0.75%)，Ⅶ 类 9 指蹼 (1.70%)，Ⅷ 类 15 指蹼 (2.83%)。结论 束带综合征手畸形表现复杂，报道了指骨发育不全、缩窄带和并指的发病情况与畸形分类，为该病手部畸形评估和手术治疗计划提供一定指导，也为该病手畸形严重程度分级研究提供了基础。更多还原

【Abstract】 Objective To explore the clinical manifestations of hand deformities in congenital constriction band syndrome,and enrich the understanding of clinical presentation associated with this condition and guide clinical assessment and treatment.Methods From January 2012 to January 2024,patients with constriction band syndrome admitted to our clinical and research center were reviewed.Clinical data such as age,gender,site of involvement,and associated deformities were recorded.Clinical photograph and X-ray data were used to assess the manifestations and severity of phalangeal hypoplasia,constricting bands,and syndactyly.Results A total of 53 patients were included,with 23 males (43.40%) and 30 females (56.60%),aged 0.92-17 years with a mean age of (3.92±4.01) years.Bilateral hand involvement was observed in 27 cases,isolated left involvement in 11 cases,right in 14 cases,and one case with normal hand but forearm involvement.Among 530 fingers,type Ⅰ phalangeal hypoplasia accounted for 13.02%,type Ⅱ for 28.87%,and type Ⅲ for 9.81%;21.87% of the 530 fingers had constricting band,with type Ⅰ at 6.42%,type Ⅱ at 7.74%,type Ⅲ at 4.53%,type Ⅳ at 5.28%,and multiple bands at 3.21%;among 212 involved webs,type Ⅰ syndactyly accounted for 1.89%,type Ⅱ for 8.68%,type Ⅲ for 2.26%,type Ⅳ for 3.02%,type Ⅴ for 1.70%,type Ⅵ for 0.75%,type Ⅶ for 1.70%,and type Ⅷ for 2.83%.Conclusions Hand deformities in congenital constriction band syndrome are complex.This study explores the manifestation and classification of phalangeal hypoplasia,constricting bands,and syndactyly.The outcomes would help guide the assessment and surgical treatment planning of hand deformities associated with this condition,and provide references for research on the severity grading of hand deformities.更多还原